Fajri Marindra Siregar^1,2, Sofia Mubarika Haryana³, Dyah Wulan Anggrahini⁴, Lucia Kris Dinarti⁴, Anggoro Budi Hartopo^4,*

Congenital Heart Disease, Vol.19, No.4, pp. 375-388, 2024, DOI:10.32604/chd.2024.054742 - 31 October 2024

Abstract A particular type of endogenous noncoding RNAs known as circular RNAs (circRNAs) has now become possible biomarkers for several diseases because of their stability and tissue-specific expression patterns. CircRNAs might play a role in various of biological processes. The identification of particular circRNAs dysregulated in pulmonary arterial hypertension (PAH) raises the possibility of these molecules serving as biomarkers for the disease’s early diagnosis and treatment. This review mainly summarizes the role and potential of circRNA as a future biomarker in PAH related to congenital heart disease. This study presented several potential circRNA targets as diagnostic More > Graphic Abstract

Wenjie Dong^1,2,#, Zhibin Hong^1,#, Aqian Wang², Kaiyu Jiang², Hai Zhu², Fu zhang², Zhaoxia Guo², Hongling Su^2,*, Yunshan Cao^3,*

Congenital Heart Disease, Vol.19, No.3, pp. 325-339, 2024, DOI:10.32604/chd.2024.052267 - 26 July 2024

Abstract Background: Current guidelines for managing pulmonary arterial hypertension (PAH) recommend a risk stratification approach. However, the applicability and accuracy of these strategies for PAH associated with congenital heart disease (PAH-CHD) require further validation. This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up. Additionally, new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients. Methods: This retrospective study included 126 PAH-CHD patients. Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s… More > Graphic Abstract

CHENYANG CHEN^1,*, JUAN WEN¹, WEI HUANG¹, JIANG LI^2,*

BIOCELL, Vol.47, No.11, pp. 2423-2433, 2023, DOI:10.32604/biocell.2023.044459 - 27 November 2023

Abstract Background: Pulmonary arterial hypertension (PAH) is a chronic and progressive disease that is strongly associated with dysregulation of glucose metabolism. Alterations in nuclear receptor subfamily 4 group A member 1 (NR4A1) activity alter the outcome of PAH. This study aimed to investigate the effects of NR4A1 on glycolysis in PAH and its underlying mechanisms. Methods: This study included twenty healthy volunteers and twenty-three PAH patients, and plasma samples were collected from the participants. To mimic the conditions of PAH in vitro, a hypoxia-induced model of pulmonary artery smooth muscle cell (PASMC) model was established. The proliferation… More > Graphic Abstract

Andrei George Iosifescu^1,2,*, Roxana Enache^1,3, Ioana Marinică⁴, Corina Radu², Vlad Anton Iliescu^1,2

Congenital Heart Disease, Vol.18, No.1, pp. 67-72, 2023, DOI:10.32604/chd.2023.026598 - 09 January 2023

Abstract Left main coronary compression syndrome (LMCS) may complicate pulmonary artery aneurysms (PAA), usually developed in the context of pulmonary arterial hypertension (PAH). We report the case of a 51-year-old female patient with an atrial septal defect (unsuitable for device closure) complicated by a PAA generating a 90% left main stenosis. The significant PAH held us back from immediate surgery. After specific dual PAH-targeted therapy (sildenafil and bosentan), the atrial septal defect could be closed with a unidirectional valved patch; the PAA-induced LMCS was treated by reductive arterioplasty. The postoperative course was uneventful. Follow-up showed clinical More > Graphic Abstract

Rui Jiang^1,3,*, Kaisheng Lai², Jianping Xu¹, Xiang Feng¹, Shaoye Wang¹, Xiaojian Wang³, Zhe Liu²

Congenital Heart Disease, Vol.17, No.6, pp. 675-686, 2022, DOI:10.32604/chd.2022.021626 - 11 October 2022

Abstract Background: The etiology of pulmonary arterial hypertension associated with congenital heart disease (PAHCHD) is complicated and the phenotype is heterogeneous. Genetic defects of NOTCH3 were associated with cerebral disease and pulmonary hypertension. However, the relationship between NOTCH3 mutations and the clinical phenotype has not been reported in CHD-PAH. Methods: We eventually enrolled 142 PAH-CHD patients from Fuwai Hospital. Whole exome sequencing (WES) was performed to screen the rare deleterious variants of NOTCH3 gene. Results: This PAH-CHD cohort included 43 (30.3%) men and 99 (69.7%) women with the mean age 29.8 ± 10.9 years old. The pathogenic or likely pathogenic… More >

Mohamed Saber Hafez^*, Alaa Roushdy, Dina Ezzeldin

Congenital Heart Disease, Vol.17, No.3, pp. 325-333, 2022, DOI:10.32604/chd.2022.019025 - 03 May 2022

Abstract Background: Coarctation of the aorta is a congenital heart disease that sometimes remains clinically silent until adulthood, usually presenting with arterial hypertension. It is well known that after coarctation treatment, many patients remain hypertensive despite successful repair. In this study we approached the predictors and effects of residual hypertension after successful coarctation therapy. Methods: It was a cross sectional observational study involving 50 patients who underwent Coarctation repair/angioplasty in Ain Shams university hospitals. We divided the patients into two groups (hypertensive and normotensive) and we studied their demographic data (e.g., age, gender, age at first repair,… More >

Marie Laure Yammine^#,*, Camilla Calvieri^#, Marcello Chinali, Salvatore Giannico, Giulia Cafiero, Ugo Giordano

Congenital Heart Disease, Vol.16, No.5, pp. 457-467, 2021, DOI:10.32604/CHD.2021.015896 - 03 June 2021

Abstract Background: In recent decades, aortic stenting has become a promising alternative to surgery for both native aortic coarctation and re-stenosis in children and adults. However, comparative long-term outcomes have poorly been investigated. Methods: We included 212 patients with previous aortic repair (19 ± 8.7 years) divided into 3 groups: 139 with single-time surgical repair (CoA-S group); 18 with single-time percutaneous stenting (CoA-PS group); and 55 hybrid patients with multiple aortic procedures because of re-coarctation occurrence (CoA-H group). All patients underwent 24-hour ambulatory blood pressure monitoring and trans-thoracic echocardiography. Results: After a median follow-up of 17 years after… More >

Dongdong Zheng^1,#, Chi Shen^1,2,#, Wenshi Liu¹, Wenjing Lv¹, Xiaofei Li^1,*

Congenital Heart Disease, Vol.16, No.4, pp. 373-381, 2021, DOI:10.32604/CHD.2021.015260 - 19 April 2021

Abstract Objectives: Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is the most common type of PAH and increases morbidity and mortality in patients with CHD. Right heart catheterization (RHC) is the standard method to diagnose PAH. However, RHC is an invasive and complicated method with relatively high cost. Noninvasive, feasible, and cost-efficient methods are urgently needed. The objective of this study was to evaluate three potential biomarkers of PAH-CHD: Hepatocyte growth factor (HGF), osteopontin (OPN), and suppression of tumorigenicity 2 (ST2). Methods: Plasma samples were collected from patients with CHD (n = 46) and healthy individuals (n… More >

Se Yong Jung^#, Doyoung Jung^#, Ah Young Kim, Jae Hee Seol, Jung Min Park, Jo Won Jung, Jae Young Choi^*

Congenital Heart Disease, Vol.16, No.3, pp. 233-244, 2021, DOI:10.32604/CHD.2021.014272 - 02 March 2021

Abstract Purpose: This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Materials and Methods: We conducted a retrospective study of patients with CHD-associated PAH, treated with selexipag since December 2017. Thirteen adult patients (mean age, 45.4 years; women, 77%) were treated with selexipag as add-on therapy. Baseline characteristics, World Health Organization functional class, 6-minute walking distance (6MWD) test results, N-terminal pro-B-type natriuretic peptide levels, echocardiographic data, and incidence of side effects were assessed. Results: The majority of patients (12/13, 92.3%) experienced more than one treatment-associated complication;… More >

Hongyun Ruan¹, Yigang Zhang², Ru Liu³, Xiangjun Yang¹

Congenital Heart Disease, Vol.14, No.4, pp. 645-650, 2019, DOI:10.1111/chd.12764

Abstract Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
Design: Prospective randomized controlled trial.
Setting: Tertiary cardiology center.
Patients: Adult patients with CHD‐PAH.
Interventions: Patients were randomized to Fasudil 30 or 60 mg.
Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava,… More >